OBJECTIVE: To determine whether abnormalities of the CNS are present in very young children with sickle cell anemia. STUDY DESIGN: Thirty-nine children with hemoglobin SS between the ages of 7 and 48 months were examined with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). No child had a history of clinical stroke, although 3 had a history of seizures (2 neonatal). Twenty-one patients underwent developmental testing with the Bayley or McCarthy Scales. RESULTS: The overall prevalence of CNS abnormalities in asymptomatic children was 4 of 36 (11%, confidence interval 3, 26%). One patient had a silent infarct observed on MRI and a stenotic lesion on MRA; 3 other patients had stenotic lesions on MRA. The 3 patients who had a history of seizures all had lesions consistent with infarcts on MRI. Of the asymptomatic patients who had psychometric testing, 1 of 18 was developmentally delayed. One of 3 with a history of seizures had mild developmental delay. CONCLUSIONS: Very young children with sickle cell anemia (and no history of clinical stroke) have infarction in the brain and/or stenosis of major cerebral arteries, similar to those reported in older children. These findings indicate a need for larger studies to define the incidence of CNS lesions in this age group and to determine the need for early therapeutic intervention to prevent CNS sequelae of sickle cell disease.
OBJECTIVE: To determine whether abnormalities of the CNS are present in very young children with sickle cell anemia. STUDY DESIGN: Thirty-nine children with hemoglobin SS between the ages of 7 and 48 months were examined with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). No child had a history of clinical stroke, although 3 had a history of seizures (2 neonatal). Twenty-one patients underwent developmental testing with the Bayley or McCarthy Scales. RESULTS: The overall prevalence of CNS abnormalities in asymptomatic children was 4 of 36 (11%, confidence interval 3, 26%). One patient had a silent infarct observed on MRI and a stenotic lesion on MRA; 3 other patients had stenotic lesions on MRA. The 3 patients who had a history of seizures all had lesions consistent with infarcts on MRI. Of the asymptomatic patients who had psychometric testing, 1 of 18 was developmentally delayed. One of 3 with a history of seizures had mild developmental delay. CONCLUSIONS: Very young children with sickle cell anemia (and no history of clinical stroke) have infarction in the brain and/or stenosis of major cerebral arteries, similar to those reported in older children. These findings indicate a need for larger studies to define the incidence of CNS lesions in this age group and to determine the need for early therapeutic intervention to prevent CNS sequelae of sickle cell disease.
Authors: F Daniel Armstrong; T David Elkin; R Clark Brown; Penny Glass; Sohail Rana; James F Casella; Ram V Kalpatthi; Steven Pavlakis; Zhibao Mi; Winfred C Wang Journal: Pediatrics Date: 2013-01-06 Impact factor: 7.124
Authors: Jeffrey D Lebensburger; Scott T Miller; Thomas H Howard; James F Casella; R Clark Brown; Ming Lu; Rathi V Iyer; Sharada Sarnaik; Zora R Rogers; Winfred C Wang Journal: Pediatr Blood Cancer Date: 2011-12-20 Impact factor: 3.167
Authors: Janet L Kwiatkowski; Robert A Zimmerman; Avrum N Pollock; Wendy Seto; Kim Smith-Whitley; Justine Shults; Anne Blackwood-Chirchir; Kwaku Ohene-Frempong Journal: Br J Haematol Date: 2009-06-04 Impact factor: 6.998
Authors: Kathleen J Helton; Robert J Adams; Karen L Kesler; Alex Lockhart; Banu Aygun; Catherine Driscoll; Matthew M Heeney; Sherron M Jackson; Lakshmanan Krishnamurti; Scott T Miller; Sharada A Sarnaik; William H Schultz; Russell E Ware Journal: Blood Date: 2014-06-09 Impact factor: 22.113
Authors: Courtney D Thornburg; Natalia Dixon; Shelly Burgett; Nicole A Mortier; William H Schultz; Sherri A Zimmerman; Melanie Bonner; Kristina K Hardy; Agustin Calatroni; Russell E Ware Journal: Pediatr Blood Cancer Date: 2009-05 Impact factor: 3.167